Musculoskeletal pain in schoolchildren across puberty: A 3-year follow-up study

BACKGROUND: Chronic Musculoskeletal Pain (MSP) in children can be due to non-inflammatory conditions, such as the benign joint hypermobility syndrome (BJHS) or idiopathic MSP (IMSP). Aim of the study was to evaluate type and persistence of MSP in a cohort of schoolchildren with MSP followed for 3 years, in order to identify the main risk factors. METHODS: Healthy schoolchildren, aged 8-13 years, underwent a general and rheumatologic examination, focusing on presence of chronic MSP, defined as continuous or recurrent pain lasting more than 3 months and heavily interfering with daily life activities, presence of generalized joint hypermobility, the body mass index and the pubertal stage. All symptomatic subjects were re-evaluated 3 years later with the same methods. RESULTS: Seventy of the 88 symptomatic subjects of the initial cohort of 289 were re-evaluated 3 years later. Of these, 38 (54.3 %) still presented MSP, including 19 with BJHS and 19 with IMSP. Main symptoms were lower limbs arthralgia and myalgia. MSP persisted more in females than in males (p = 0.038) and in pubertal rather than pre-pubertal subjects (p = 0.022); these subjects recovered significantly more both from BJHS (p = 0.004) and IMSP (p = 0.016). Gender did not influence the distribution of MSP according to pubertal stage. CONCLUSIONS: Female gender, BJHS and pubertal stage are important risk factors for persistence of MSP. Further studies are needed to evaluate the natural history of MSP towards adulthood and the role of the pubertal age

Spermatogenesis in young adult patients with beta-thalassaemia major long-term treated with desferrioxamine.

Since the introduction of hypertransfusion and intensive iron chelation therapy, patients with homozygous beta-thalassaemia major (TM) achieve adulthood. Many patients grow and develop normal hoping for marriage and to have a family. Therefore the question of fertility potential in this adult group of TM patients has become paramount. We report the semen parameters, the endocrine functions and serum zinc levels in 12 young adult TM patients. Their mean age was 24.8 years. Six patients (50%) had a normal sperm count, motility and morphology. While the remaining patients had oligospermia (sperm concentration <20 x 10(6)/ml) and/or asthenospermia (motility <40%). Basal serum gonadotrophins [LH and FSH], total and free testosterone and serum zinc did not differ significantly from those found in 13 normal adults with comparable testicular size. At the time of the study serum ferritin levels ranged from 240 to 3055 ng/ml (mean 1139 ng/ml). No correlations were found between semen parameters, serum total and free testosterone, plasma zinc, serum ferritin and seminal parameters. Nevertheless we observed that serum ferritin levels were lower (mean 543 ng/ml) in TM patients with abnormal seminal parameters (count and motility) compared to TM patients with normal seminal parameters (mean serum ferritin 1276 ng/ml; p<0.01). In conclusion, impairment of semen parameters may be a negative effect of intensive chelation therapy. Clearly, further investigations are required to evaluate if these adverse effects can be reduced or prevented, and if the existing spermatogenesis damage is reversible

Dry synovitis, a rare entity distinct from juvenile idiopathic arthritis

BackgroundDry synovitis (DS) is a rare entity as only a few cases have been reported to date. We describe the clinical features, radiological manifestations and course of DS in comparison with rheumatoid factor negative polyarticular juvenile idiopathic arthritis (RFneg-polyJIA).MethodsWe performed a multicenter retrospective collection of data of DS patients who presented with progressive joint limitations without palpable synovitis, absence of elevated acute phase reactants, negative ANA and RF, and imaging showing joint and/or osteochondral involvement. For comparative purposes, we included a cohort of RF neg-polyJIA patients.ResultsTwelve DS patients, 8F/4 M, with mean age at onset of 6.1 years, were included. Presenting signs comprised delayed motor development, functional limitations and/or progressive stiffness. Clinical examination showed symmetric polyarticular involvement with variable muscular atrophy. MRI showed mild, diffuse synovial involvement, without effusion. With time, signs of progressive osteochondral damage became evident, despite treatment. All patients were treated with low-dose corticosteroids and methotrexate. Anti-TNF agents were prescribed in five. The response was variable with limited joint mobility in 11/12, and need of joint replacement in 2. In comparison with a cohort of RFneg-polyJIA, DS patients presented higher number of joint involved (p = 0.0001) and contractures (p = 0.0001), less swelling (p = 0.0001) and prolonged diagnostic delay (p = 0.0001).ConclusionDS represents a unique juvenile-onset arthropathy, distinct from polyarticular JIA. Awareness among pediatricians is essential for early recognition and proper treatment. Further studies, including synovial pathology, immunology and genetics may contribute to a better understanding of this rare disorder of childhood